Objective To investigate the clinical features, treatment and prognostic factors of rhabdomyosarcoma (RMS) in nasal cavity and paranasal sinus.Methods Clinical data of 15 patients with RMS of nasal cavity and paranasal sinuses admitted to our department from Jan 2012 to Jan 2022 were retrospectively analyzed. Most of the 15 patients were adolescents. The pathological diagnosis was embryonal RMS in 12 cases, alveolar in 3. As for the treatment strategies, 1 case received chemotherapy alone, 3 received operation and postoperative chemotherapy, 7 underwent surgery and postoperative chemoradiotherapy, and 4 received preoperative chemotherapy and surgery and postoperative chemoradiotherapy. SPSS 26.0 statistical software was used for descriptive statistical analysis. Kaplan-Meier method was used to evaluate the relationship between clinical data, pathological features, tumor stage and prognosis.Results Follow-up of 3 to 113 months after treatment revealed no recurrence in 6 cases, locoregional recurrence in 4 and systemic recurrence in 5. During follow-up period, 8 patients survived and 7 died. Survival analysis showed that the overall survival rates for 1 year and 5 years were calculated as 93.3% and 43.6%. Pathological type, regional lymph node metastasis, distant metastasis, treatment method, intergroup rhabdomyosarcoma study (IRS) stage were correlated with the prognosis of nasal and paranasal sinus RMS(P＜0.05).Conclusions RMS in the nasal cavity and paranasal sinus is rare in clinic, with characteristics of easily invading the orbit and skull base, high recurrence rate and mortality. Multiple-disciplinary treatment is effective, operation combined with adjuvant chemoradiotherapy can reduce the recurrence rate and improve the prognosis. To improve the clinical and pathological understanding, conduct molecular and genomic studies, and find new therapeutic targets are expected to improve the life quality and survival rate of RMS patients.